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Medicine - Year 2 > The haematological system and skin - Coagulation and the bone marrow in health and disease > Flashcards

The haematological system and skin - Coagulation and the bone marrow in health and disease Flashcards

1
Q

Thrombophilias - who should be screened

A

Patients with thrombosis who are young

Positive family history

Thrombosis in an unusual site

Recurrence

Females with recurrent foetal loss

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2
Q

Inherited thrombophilias

A

Factor V Leiden

Protein C deficiency

Protein S deficiency

Antithrombin III deficiency

Prothrombin mutation

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3
Q

Acquired thrombophilia

A

Oestrogen therapy, contraceptive pill

Malignancy

Pregnancy and puerperium

Lupus anticoagulant (antiphospholipid) syndrome

Raised plasma homocysteine (may also be inherited)

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4
Q

Lupus anticoagulant (antiphospholipid) syndrome - clinical features

A

CLOT

Clots: arterial/venous thrombosis

Lived reticularis

Obstretic loss: recurrent miscarriages

Thrombocytopenia

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5
Q

Lupus anticoagulant (antiphospholipid) syndrome - laboratory features

A

Prolonged APTT

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6
Q

Lupus anticoagulant (antiphospholipid) syndrome - Management

A

Lifelong anticoagulation

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7
Q

Management of warfarin - major bleeding

A

Stop warfarin

Administer IV vitamin K

Administer prothrombin complex ( or fresh frozen plasma if prothrombin complex unavailable)

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8
Q

Management of warfarin - minor bleeding

A

Stop anticoagulants

Administer IV vitamin K

Repeat INR after 24 hours, may need further vitamin K

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9
Q

Management of warfarin - no bleeding with INR > 8

A

Stop anticoagulants

Administer IV or oral vitamin K

Repeat INR after 24 hours

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10
Q

Management of warfarin - no bleeding with INR between 5-8

A

Withhold 1-2 doses of warfarin and restart at reduced dose

Review maintenance dose of warfarin

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11
Q

Heparin - management of over anticoagulation/bleeding

A

Stop heparin – short half-life, may be sufficient

Local measures e.g., apply pressure

Consider tranexamic acid

If bleeding, consider protamine sulphate

Look for cause e.g., incorrect dose, new renal failure

Before restarting check risk: benefit ratio

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12
Q

DOAC - management of anticoagulation/bleeding

A
  • Stop DOAC
  • Local measures e.g., apply pressure
  • Consider tranexamic acid

Idracruzimab reveral agent for dabigatran

  • Antidote to Xa inhibitors not yet available. In the meantime, in life threatening bleeding consider prothrombin complex concentrate
  • Look for cause
  • Before restarting check risk:benefit ration
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13
Q

Warfarin: risk factors for bleeding

A
  • Elderly
  • Renal failure
  • Liver failure
  • Recurrent falls
  • Platelet of NSAIDs use
  • Alcoholism
  • Cancer
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14
Q

Leucocytosis - causes

A

Primary
- Leukaemia/ lymphoma/ myeloproliferative disorders

Secondary
- infection
- Inflammation
- Infarction
- Tumour

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15
Q

Thrombocytosis - causes

A

Primary - essential thrombocythemia

Secondary
- Infection
- Inflammation
- Infarction
- Tumour

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16
Q

Essential thrombocythemia - aetiology and pathophysiology

A

JAK2 mutation in 50% cases

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17
Q

Essential thrombocythemia - clinical features

A

Asymptomatic - at least 30%

Thrombosis (arterial/venous)

Headaches, visual disturbances

Excessive haemorrhage may occur spontaneously or after trauma or surgery

Pruritis and sweating are uncommon

Gout (raises uric acid)

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18
Q

Essential thrombocythemia - laboratory features

A

Raised platelet count

Raised red cell and/or white cell in 30%

Blood film - platelet anisocytosis with circulating megakarocyte fragments. Auto infraction of the spleen may case target cells, Howell-Jolly bodies

JAK2 mutation

Raised serum uric acid

Serum LDH normal

Bone marrow - hyper cellular + increased megakarocytes

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19
Q

Essential thrombocythemia - management

A

Aspirin only in younger patients < 40 years

Chemotherapy with hydroxycarbamide + aspirin in > 40 years

Alpha interferon (may be used in younger patients but needs injections and has side- effects - flu like symptoms)

Allopurinol for gout

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20
Q

True erythrocytosis “polycythaemia” - definition and causes

A

Definition
- Increased number of red cells

Causes
- Primary: Polycythaemia rubra vera
- Secondary: low oxygen e.g., cold, tumours, doping, high affinity haemoglobin

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21
Q

Apparent erythrocytosis - defenition and causes

A

Definition
- Reduced plasma volume

Causes
- Smoking
- Overweight
- Alcohol excess
- Medications e.g., diuretics

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22
Q

Polycythaemia rubra vera - aetiology and pathophysiology

A

Mutations of JAK2 in > 995% cases

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23
Q

Polycythaemia rubra vera - clinical features

A
  • Aquagenic pruritus (itchiness after hot bath especially)
  • ‘Ruddy complexion’/plethora/redness
  • Teaches and visual disturbances
  • Thrombosis (arterial/venous)
  • Haemorrhage especially GI
  • Splenomegaly
  • Hyperviscosity symptoms: chest pain, myalgia, weakness, headache, blurred vision, loss of concentration
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24
Q

Polycythaemia rubra vera - Laboratory features

A
  • Raised hb, haemoatocrit and red cell count
  • Raised white cells and/or platelets (in 75% people)
  • JAK2 mutation
  • Raised serum uric acid
  • Serum LDH normal or slightly raised
  • Low serum EPO (to prevent further erythrocytosis)
  • Hypercellular bone marrow with deplete iron stores
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25
Q

Polycythaemia rubra vera - management

A

Venesection +/- chemotherapy with oral hydroxycarbamide + aspirin

Plasmapheresis for hyperviscosity syndrome

PPI for patients with indigestion or history of GI bleeding

Allopurinol to prevent hyperuricemia

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26
Q

Polycythaemia rubra vera - prognosis

A

Development of myelofibrosis (in up to 30%

AML (in ups o 5%, not increased by hyrdoxycarbamide)

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27
Q

Thrombocytopenia - causes

A

Underproduction
- Drugs affect stem cell
- Liver failure
- Part of pancytopenia due to marrow failure

Peripheral destruction
- Autoimmune (ITP)
- Hypersplenism
- Drugs
- Infection/inflammation/ sepsis

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28
Q

Low blood counts caused by reduced production - causes

A

Myeloma

Myelodysplasia

Metastatic malignancy

Myelofibrosis

Leukaemia

Lymphoma

Aplastic anaemia

Haematinic deficiency

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29
Q

Immune thrombocytopenia purpura (ITP) - definition

A

Autoimmune disease of Unkown cause where the number of platelets is reduced

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30
Q

Immune thrombocytopenia purpura (ITP) - clinical features

A

Isolated thrombocytopenia:

  • On incidental finding or
  • Features of purpura or
  • Other minor bleeding
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31
Q

Immune thrombocytopenia purpura (ITP) - management

A

Oral prednisolone - patients with a platelet of <30 × 10^9/L or a platelet count >30 × 10^9/L and features of minor bleeding/high risk

Splenectomy - refractory cases

Avoid platelet transfusions in the absence of life-threatening bleeding

32
Q

Myelodysplasia (myelodysplastic syndrome) - definition

A

Haematopoietic stem cell malignancy where there is abnormal maturation as well as proliferation of cells in bone marrow.

It is characterised by peripheral blood cytopenia

33
Q

Myelodysplasia (myelodysplastic syndrome) - Aetiology and pathogenesis

A

May be primary or secondary, as. consequence of a previous chemotherapy/radiotherapy

34
Q

Myelodysplasia (myelodysplastic syndrome) - laboratory findings

A

Macrocytic anaemia

Pancytopenia

Blood film - Active, cellular marrow

35
Q

Myelodysplasia (myelodysplastic syndrome) - differential diagnosis

A

Other causes of anaemia must be excluded

Myelofibrosis

Aplastic anaemia

36
Q

Myelodysplasia (myelodysplastic syndrome) - management

A

Supportive care with red cell or platelet transfusion and antimicrobials may be required

Erythropoietin

Iron chelation theory to prevent iron overload

Chemotherapy

Allogenic stem cell transplant (may cure younger patients)

37
Q

Aplastic anaemia - definition

A

Chronic pancytopenia associated with hypoplastic bone marrow

38
Q

Aplastic anaemia - aetiology and pathogenesis

A

May be acquired or congenital (e.g., Fanconi’s anaemia), dyskeratosis congenital

39
Q

Aplastic anaemia - epidemiology

A

May occur at any age, in either sex

40
Q

Aplastic anaemia - Clinical features

A

Onset rapid (over few days) or slow (over weeks or months

Liver, spleen and lymph nodes not enlarged

41
Q

Fanconi’s anaemia - genetic inheritance

A

Autosomal recessive

42
Q

Fanconi’s anaemia - clinical features

A
  • Presents in childhood
  • Pigmentation abnormalities
  • Hearing defects
  • Renal abnormalities
  • Genital abnormalities
  • Solid tumours
  • Short stature
43
Q

Aplastic anaemia - laboratory findings

A

Normocytic anaemia or mild microcytic with a low reticulocyte

Pancytopenia

Blood film- hypo plastic with > 75% fat spaces (“empty” bone marrow)

44
Q

Aplastic anaemia - management

A

Immunosuppression

Androgens

Stem cell transplantation is a cure in severe cases

Haemopoietic growth factors, granulocyte colony-stimulating factor may raise neutrophil count temproily

Blood product support

45
Q

Myelofibrosis - aetiology and pathophysiology

A

Malignant proliferation of reticulin fibres in bone marrow causing anemia,

46
Q

Myelofibrosis - epidemiology

A

Sexes affected equally

> 50 years

47
Q

Myelofibrosis - clinical features

A

Frequent - fever, weight loss, pruritus, hepatomegaly and night sweats

Less common - gout, bone and joint pain

Late stages - abdominal swelling, ascites and bleeding from oesophageal varicose occur

48
Q

Myelofibrosis - laboratory features

A

Normochromic normocytic anaemia

Leucocytosis and thrombocytosis occur early, and later leucopenia and thrombocytopenia

Blood film: red cell poikilocytes with teardrop forms

JAK2 mutation in 50% cases

LDH raise (unliked in PRV and ET)

LFTs are often abnormal because of extramedullary haemopoiesis

“Dry tap” on attempt of bone marrow aspiration

49
Q

Myelofibrosis - management

A
  • Chemotherapy with hydroxycarbamide
  • JAK2 inhibitors
  • Thalidomide - improves marrow function and reduces spleen size
  • Supportive therapy with red cell transfusions, folic acid and occasionally platelett transfuion
  • Iron chelation to prevent iron overload
  • Splenectomy or splenic irradiation
  • Allogenic stem cell transplantation: a cure for younger patients
50
Q

Myelofibrosis - prognosis

A

Can transform into acute lymphoblastic leukaemia

51
Q

Hyposplenism - causes

A

Splenectomy

Auto-infarction e.g., sickle cell disease

infiltration - metastatic malignancy

Under-function - coeliac disease

52
Q

Neutrophilia - causes

A

Primary
- Myeloproliferative disorder e.g., chronic myeloid leukaemia

Secondary
- Bacterial infection
- Inflammatory conditions
- Burns
- Cigarette smoking
- Steroids
- G-CSF
- Solid tumour

53
Q

Lymphocytosis - causes

A

Viral infection e.g., EBV

Hyposplenism

TB

Brucellosis

Chronic lymphocytic leukaemia (CLL)

Lymphoma with ‘spill over’

54
Q

Eosinophilia - causes

A

Allergic reactions

Vasculitis

Drus

Worm infestations

Cancer (especially solid tumours and lymphoma)

55
Q

High grade lymphomas - characteristics

A
  • Short history
  • Grows quickly
  • Patiently usually symptomatic
  • Treatment always required immediately
  • Potentially curable
  • Treatment intensive chemotherapy
  • Once chance to cure (or maybe two)
56
Q

Low grade lymphomas - characteristics

A
  • Often longer or ‘no’ history
  • Grows slowly
  • Patients often asymptomatic
  • Treatment often not required (watch and wait)
  • A lifelong illness
  • Treatment generally less intensive chemotherapy
  • Can usually treat again and again…
57
Q

Lymphomas - clinical features

A
58
Q

Hodgkin lymphoma - epidemiology

A

Males > females

Most common in young adults and over 60s

59
Q

Hodgkin lymphoma - histological classification

A

Characterised by the presence of reed-sterner cells

60
Q

Hodgkin lymphoma - types

A
  1. Classical
    - Nodular sclerosis
    - Mixed cellularity
    - Lymphocytic-rich
    - Lymphocytic-deplete
  2. Nodular lymphocyte-predominant
61
Q

Hodgkin lymphoma - clinical features

A

Lymphadenopathy - painless, alcohol may precipitate pain

‘B’ symptoms - fever, night sweats and weight loss (unexplained, > 10% in 6 months)

Mediastinal mass

Pruritus

Splenomegaly/hepatosplenomegaly

Malaise

Fatigue

Extra nodal disease - lung, CNS, skin and bone involvement

62
Q

Hodgkin lymphoma - investigations

A

Bloods - FBC - U&eS, Bone profile, LDH, Uric acid, ESR

Imaging
- CXR
- PET CT: used in staging of disease
- CT neck, chest, abdomen and pelvis
- MRI

Additional
- Lumbar puncture and CSF analysis: in those with suspected CNS disease
- Echocardiogram
- Pulmonary function tests
- Bone marrow biopsy

63
Q

Hodgkin lymphoma - laboratory features

A
  • Anaemia (normochromic, normocytic; AIHA can occur)
  • Leucocytosis
  • Raised ESR
  • Raised LDL
  • Abnormal LFTs
64
Q

Hodgkin lymphoma - staging used

A

Ann Arbour staging

65
Q

Describe Ann Arbor staging

A

Stage I - single lymph node region

Stage 2 - two or more lymph node regions on the same side of the diaphragm

Stage 3 - two or more lymph node region above and below the diaphragm

Stage 4 - widespread disease; multiple organs with out with lymph node involvement

A: absence of B symptoms
B: fever, nights sweats and weight loss

66
Q

Hodgkin lymphoma - management

A

Chemotherapy

67
Q

Non-hodgekin lymphoma - histological classification

A

Characterised by no reed-sternberg cells

68
Q

Non-hodgkin lymphoma - presentation

A

Lymphadenopathy

‘B’ symptoms - fever, night sweats and weight loss (unexplained, > 10% in 6 months)

Pruritus

Splenomegaly

Hepatomegaly

69
Q

Non-hodgkin lymphoma - investigations

A

Bloods – FBC, U&Es, LFTs, Bone profile, LDH, Uric acid, ESR,

Imaging
- CXR
- PET CT: Used in the staging of disease
- CT neck, chest abdomen and pelvis
- MRI brain
- Bone scan

Additional
- Lumbar puncture and CSF analysis: in those with suspected CNS disease
- Bone marrow aspirate and biopsy

70
Q

Non-hodgkin lymphoma - investigations - laboratory features

A

Anaemia or pancytopenia

Peripheral blood lymphocytosis

Paraprotein (especially in lymphoplasmacytic lymphoma) or hypogammaglobinaemia

Serum LDH raised in more aggressive forms

Raised serum B2-microglobulin

71
Q

Non-hodgkin lymphoma - staging used

A

Ann arbour staging

72
Q

Non-hodgekin lymphoma - high-grade lymphomas examples

A

Diffuse large b-cell lymphoma

Burkitt lymphoma

73
Q

Burkitt’s Lymphoma - appearance on biopsy

A

‘Starry’ appearance

74
Q

What type of non-hodgekin lymphoma is associated with Helicobacte Pylorir?

A

Gastric MALT (mucosa-associated lymphoma tissue

75
Q

What types of non-hodgekin lymphoma is associated with EBVr?

A

Burkitt’s lymphoma

(AIDS-related CNS lymphoma)

76
Q

What types of Non-hodgekin lymphoma is associated with hepatitis C

A

Large B-cell lymphoma

(Splenic marginal zone lymphoma)

77
Q

Non-hodgekin lymphoma - management

A

Chemotherapy

Medicine - Year 2 (27 decks)

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